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What's New in Cerebral PalsyIt is becoming more
evident that difficult labors and post-natal asphyxia may be
the result of defective infants rather than the cause of
them.
THE CARE OF THE HANDICAPPED CHILD is one of the major
medical and social issues facing our society today. Huge
strides have been made in technology, treatment, and
recognition of psycho social problems, leading to the
ultimate goal of integrating these individuals as useful and
productive members of society. This progress is well
illustrated in our current approach to cerebral palsy.
Etiology
Cerebral palsy was first described by William John Little of
England in 1843. He related this condition to asphyxia from
birth problems. It has taken many years and intensive
epidemiological studies to overcome the popular notion that
the major cause relates to the birth process. A significant
prospective study was carried out in this country in the
1960's and 1970's in which 50,000 individuals were followed
from the onset of pregnancy to age seven. This study
revealed that ten percent or less of cerebral palsy could be
attributed to problems of oxygenation at birth. It is
becoming more evident that difficult labors and post natal
asphyxia may be the result of defective infants rather than
the cause of them. More and more of these babies are found
to have congenital malformations of the brain on CT scan.
Prematurity is now recognized as a major risk factor.
Although the majority of premature infants develop normally,
the risk of cerebral palsy is 27 times higher in infants who
weigh less than three pounds at birth compared to those who
weigh five pounds or more. The cerebral arterial system is
very fragile in this group and transitory increases in blood
pressure often cause hemorrhage into the region around the
third ventricle. If this hemorrhage is extensive, it results
in periventricular leucomalacia in the region of the
internal capsular leg fibers causing spastic diplegia.
In some instances, it is possible to identify the etiology
of C.P. such as in genetic syndromes, congenital
malformations, and in utero infections. There is a high risk
factor in premature infants and those with prolonged low
Apgar scores. However, in the majority of cases, no specific
etiology can be found. We can probably diminish the
incidence of cerebral palsy by decreasing the occurrence of
prematurity and perinatal asphyxia.
Incidence
The incidence of cerebral palsy in the Western World is
approximately two per thousand live births. This has not
diminished despite more wide spread use of fetal monitoring
and the proliferation of technologically advanced neonatal
intensive care units. Many high risk infants who formerly
died are now surviving with cerebral palsy, and many who
formerly survived with cerebral palsy are now emerging
neurologically intact. In fact, the incidence of cerebral
palsy is lower in Third World countries because fewer of
their high risk infants survive.
Despite the failure of medical progress to diminish the
incidence of cerebral palsy, there have been profound
changes in the spectrum of this condition. With the
elimination of Rh incompatibility, the occurrence of
athetoid quadriplegia has greatly diminished. With improved
neonatal care, there are fewer globally involved patients
attributable to hypoxia. We are seeing more spastic
diplegics secondary to prematurity. The pendulum appears to
be swinging toward children with less severe and more
treatable types of cerebral palsy.
Treatment
Communication, independence in self care activities, and
mobility are considered by patients to be more critical to
their function than ambulation. Because of the multiplicity
of problems associated with C.P.. a multi disciplinary
clinic is the optimal setting for treatment of this
condition. The Newington Children's Hospital C.P. Clinic
includes orthopaedics, neurology, pediatrics, rehabilitation
services, orthotics, and social services with accessibility
to speech and hearing, ophthalmology, psychology, and
educational evaluations. It is comforting for C.P. patients
and their parents to have all their needs addressed at one
time and in one place by experienced and caring
professionals.
Communication problems in C.P. are receiving great
attention. Recent advances in computer technology have
enabled many children to write or actually speak through
machines with programmed responses, utilizing the most
minimal physical abilities such as eye movements and
breathing. This has opened new vistas to the cognitively
adept patient who is locked into a severely handicapped
body. We can predict that this group of individuals will be
much more productive members of society.
Orthotics have been widely used to prevent deformity and
enhance function. Light weight plastic materials improve
cosmesis and are less burdensome to the patient. Very few
above knee orthoses are used today. Knee joint extension is
achieved by utilizing the plantar flexion knee extension
couple of floor reaction orthoses. More articulated ankle
AFO's are being prescribed to allow a closer approximation
to normal gait. New technology produces customized seating
devices for the severely handicapped which lessen the risk
of skin irritation and allow maximal upper extremity
function from the upright position.
The role of physical therapy is becoming more defined. There
are some recent prospective studies showing no difference
between groups receiving early P.T. and those with no P.T.
Or early intervention programs. However, P.T. is very useful
for parental guidance, addressing equipment needs,
prevention of deformity, post operative rehabilitation, and
maintenance of operative gains. Selective dorsal rhizotomy
is the latest neurosurgical procedure being utilized in the
treatment of a segment of the
C.P. population. Increased muscle tone results from a
combination of over-stimulation of the anterior horn cell
from the muscle spindle and diminished inhibitory control
from the cerebral cortex. By resecting the sensory fibers
within each lumbar and upper sacral dorsal root showing
hyperactivity on intraoperative EMG exam and sparing those
showing a normal response, muscle tone is diminished, but
not lost. No loss of peripheral sensation has been reported
and the early results have bee encouraging in the C.P.
patient whose function is being hampered by overwhelming
tone or spasticity. It is contra-indicated in patients with
athetosis, rigidity, and underlying weakness. It will not
correct contractures and angular deformities already
present.
Medications play a very limited role in treatment except for
seizure medications. Valium is quite useful in managing
perioperative spasm. No agent has been shown to have long
term tone reducing ability.

The Center for Motion Analysis at Connecticut Children's
Medical Center is world-renowned for its involvement with
the study of cerebral palsy problems.
Gait analysis is the most exciting new modality to arrive on
the scene in many years. In a state of the art gait analysis
laboratory, such as the one at Connecticut Children's
Medical Center, three dimensional kinematics, EMG's, and
kinetic or force and moment analyses can be produced through
appropriate computer software in a very short period of
time. This allows the experienced gait analyst to assess the
physical exam, video analysis, along with the above studies
and formulate an appropriate, objective plan of treatment
for the individual being evaluated. Post operative studies
document the results of surgery and allow an objective
assessment of various treatment methods. The gait laboratory
at Connecticut Children's Medical Center is world-renowned
for its involvement with the study of cerebral palsy
problems.
Finally, orthopaedic surgery is still necessary to correct
contractures, correct bony deformities, and re-balance
abnormal muscle forces producing gait deviations. Operative
procedures have evolved. Muscle releases often resulted in
functional weakness or over correction, producing a
deformity opposite to the one initially addressed. Hence, we
now perform more muscle lengthening and split tendon
transfers to achieve balance around a joint. Kinetic studies
in the gait laboratory demonstrate the importance of the
ilio-psoas and the gastrocnemius as power sources for the
swing phase of gait. Thus we are more apt to spare these
muscles or to perform very minimal lengthening. Osteotomies
are secured with stable infernal fixation to avoid
postoperative immobilization and functional regression. Hips
are not allowed to dislocate and progressive scoliosis is
surgically stabilized to maintain sitting ability and
optimize function.
We have a better understanding of the interdependence of
the joints of the lower extremity and the severe psycho
social trauma of multiple hospital admissions. Most of the
involved lower extremity muscles span two joints, i.e.
gastrocnemius, hamstrings, rectus femoris, and psoas. Thus,
operating upon one joint will produce an effect on the
adjacent joints. Because of this, we now attempt to perform
all our corrective surgery, bony and/or soft tissue, during
one surgical episode, often utilizing two teams of surgeons
for bilateral cases to shorten the operative time. We can
balance all the joints of the lower extremity, avoid
multiple hospital admissions, and allow for an uninterrupted
period of rehabilitation when the entire limb can be treated
definitively. This also eliminates multiple periods of
functional regression that normally follow each surgical
procedure.
The C.P. patient and his family are best served by a
multidisciplinary clinic where all needs and problems,
physical and emotional, can be addressed at one time by one
group.
Summary
The etiology of cerebral palsy appears to be less related to
hypoxia at birth than was once thought. The type of
involvement has been shifting from the severe quadriplegics,
such as seen with Rh incompatibility, to the more treatable
spastic diplegics, often seen in those born prematurely.
Despite improved perinatal care, the incidence of C.P. has
not declined, but actually may be rising in the Western
World.
The priorities of C.P. patients are more widely
recognized and society is becoming more responsive to their
needs. New light weight orthoses enhance functional
capacity. Seating devices and computerized speech aides have
become more sophisticated. Selective dorsal rhizotomy shows
initial promise in helping to control overwhelming tone.
Orthopaedic surgeons are recognizing the need to balance
joints and eliminate multiple hospital admissions by
performing all surgical procedures at one time. Gait
analysis is of great value for preoperative planning and
postoperative assessment. And, lastly, the C.P. patient and
his family are best served by a multidisciplinary clinic
where all needs and problems, physical and emotional, can be
addressed at one time by one group.
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